ObjectiveTo investigate the diagnostic accuracy and grading capability of ultrasound-derived fat fraction （UDFF）， controlled attenuation parameter （CAP）， and hepatic/renal ratio （HRR） in assessing hepatic steatosis in metabolic associated fatty liver disease （MAFLD） with magnetic resonance imaging-proton density fat fraction （MRI-PDFF） as the gold standard. MethodsA total of 150 patients with MAFLD who attended The First Hospital of Hebei Medical University from January 2023 to December 2024 were enrolled， and 148 healthy volunteers were recruited. All subjects underwent MRI-PDFF， UDFF， CAP， and HRR examinations. Hepatic steatosis was graded based on MRI-PDFF （S0：148 cases； S1：92 cases； S2：21 cases； S3：37 cases）， and the MAFLD patients with different grades of hepatic steatosis were compared in terms of UDFF， CAP， HRR， and clinical features. A one-way analysis of variance was used for comparison of normally distributed continuous data between multiple groups and the Tukey HSD test was used for further comparision between two groups； the Kruskal-Wallis H test was used for comparison of non-normally distributed continuous data between multiple groups， and the Mann-Whitney U test was used for further comparison between two groups； the chi-square test was used for comparison of categorical data between groups. The Spearman correlation analysis was used to investigate the correlation between UDFF， CAP， HRR， and MRI-PDFF in different grades of MAFLD； the receiver operating characteristic （ROC） curve was used to investigate the efficacy of UDFF， CAP， and HRR in the diagnosis of different degrees of hepatic steatosis in MAFLD； the Bland-Altman difference plot was used to analyze the consistency between UDFF and MRI-PDFF in different degrees of hepatic steatosis in MAFLD. ResultsUDFF measurement gradually increased with the increase in the grade of fatty liver （H=201.52，P0.001）. The Spearman correlation analysis showed that there was a strong correlation between any two indicators of UDFF， CAP， HRR， and MRI-PDFF in S1， S2， and S3 MAFLD （all P0.001）， with the strongest correlation between UDFF and MRI-PDFF （r_s1=0.884，r_s2=0.962，r_s3=0.929， all P0.001）. The ROC curve analysis showed that UDFF had a larger area under the ROC curve （AUC） than CAP and HRR in the graded diagnosis of S1 and S3 （all P0.05）， while in the diagnosis of S2 MAFLD， UDFF had a significantly larger AUC than HRR （P0.05） and a similar AUC to CAP （P0.05）. The Bland-Altman difference plot showed good consistency between UDFF and MRI-PDFF in different degrees of hepatic steatosis in MAFLD. ConclusionCompared with CAP and HRR， UDFF can accurately measure liver fat content and has good efficacy in identifying varying degrees of hepatic steatosis in MAFLD.

Objective:To analyze the clinicopathological characteristics and prognostic factors of patients with Castleman disease (CD).Methods:A retrospective case-series study was conducted. A total of 85 patients newly diagnosed with CD in the First Affiliated Hospital of Air Force Medical University between July 2007 and August 2024 were collected. Their clinical characteristics and prognostic factors were analyzed.Results:Among the 85 patients, 45 had unicentric Castleman disease (UCD) and 40 had multicentric Castleman disease (MCD). In the UCD group, females were more commonly affected (64.4%, 29/45), with a median age of onset of 39 years. The primary lesions were mainly located in the retroperitoneum, neck, abdomen, and axilla; and the hyaline vascular subtype was the predominant pathological type (69.4%, 25/36). In the MCD group, males were more frequently affected (62.5%, 25/40), with a median age of onset of 50 years; and the plasmacytic subtype was the main pathological type (68.2%, 15/22). Compared with UCD patients, MCD patients presented more systemic symptoms and signs [85.0% (34/40) vs. 13.3% (6/45), χ2 = 43.66, P < 0.001], splenomegaly [42.5% (17/40) vs. 2.2% (1/45), χ2 = 20.58, P < 0.001], hepatomegaly [25.0% (10/40) vs. 0 (0/45), χ2 = 10.46, P = 0.001], edema or effusion in serous cavity [67.5% (27/40) vs. 8.9%(4/45), χ2 = 31.40, P < 0.001], hematological system involvement [32.5% (13/40) vs. 0 (0/44), χ2 = 16.92, P < 0.001], and renal involvement [22.5% (9/40) vs. 2.3%(1/44), χ2 = 6.36, P = 0.012]. Laboratory findings showed that the levels of hemoglobin and albumin in MCD patients were lower than those in UCD patients, while white blood cell count in MCD patients was higher than that in UCD patients. Additionally, MCD patients exhibited elevated levels of C-reactive protein, interleukin-6, vascular endothelial growth factor, erythrocyte sedimentation rate, and ferritin compared to UCD patients (all P < 0.05). Among UCD patients, 40 cases underwent simple surgical resection, with no deaths during follow-up and the 5-year overall survival (OS) rate of 100.0%; among MCD patients, 34 cases received chemotherapy, 4 received siltuximab, 3 died during follow-up with a 5-year OS rate of 87.5%; and there was no statistically significant difference in OS between the MCD and UCD groups ( χ2 = 3.67, P = 0.055). Among MCD patients, the OS of those with renal involvement (9 cases) was worse than that of those without renal involvement (31 cases) ( χ2 = 8.39, P = 0.004). Conclusions:CD is a highly heterogeneous disorder. Surgical resection is the primary treatment for UCD, with a favorable prognosis. Chemotherapy is the main treatment for MCD, with a relatively poor prognosis.

Objective:To evaluate the utility of pulse oximetry-derived parameters—specifically, the pulse oximetry plethysmographic waveform area under the curve (POP AUC) and the peripheral perfusion index (PPI)—in assessing disease severity and predicting prognosis in patients with sepsis. Methods:In this prospective descriptive study, 68 patients with sepsis were categorized based on illness severity into septic shock and non-shock groups, and by 28-day outcome into survival and non-survival groups. POP AUC, PPI, and lactate (Lac) levels were recorded at 0, 24, 48, 72, and 96 hours after admission. APACHEⅡ and SOFA scores were calculated within the first 24 hours. The prognostic value of these parameters was evaluated. Results:Significant differences were observed between the septic shock and non-shock groups in POP AUC, PPI, Lac (all P < 0.05 except at 96 h), APACHEⅡ, and SOFA scores (all P < 0.05). These differences were most pronounced at admission: POP AUC0 (2475.1 ± 899.0) vs. (4260.3 ± 1028.5), PPI 0 (0.78 ± 0.74) vs. (3.13 ± 2.18), Lac 0 (4.95 ± 4.32) vs. (2.07 ± 1.55), APACHE Ⅱ (16.78 ± 5.59) vs. 11.82 ± 4.89), and SOFA (8.89 ± 3.25) vs. (5.06 ± 2.60). Optimal prognostic cut-off values were 2741.43 for POP AUC, 0.97 for PPI, 2.05 for Lac, 12.5 for APACHEⅡ, and 5.5 for SOFA. ROC curve analysis showed that at 24 hours, POP AUC and PPI had significantly larger AUC values than Lac ( P < 0.05), while no significant differences were found among other parameters. Significant differences between non-survivors and survivors were also found in POP AUC, PPI (at 0, 24, and 48 h), APACHE II, and SOFA (all P < 0.05). No significant differences were observed in PPI (72 h and 96 h) or Lac between the two outcome groups. Conclusions:POP AUC and PPI, as derived from pulse oximetry, are non-inferior to Lac, SOFA, and APACHEⅡ scores in evaluating disease severity and predicting 28-day mortality in sepsis patients. These parameters show promise as practical and non-invasive tools for clinical assessment in sepsis.

Objective To investigate the expression changes of interferon gene stimulating factor(STING)and E-box zinc finger binding protein 1(ZEB1)in elderly cervical cancer(CCA)patients and their correlation with human papillomavirus(HPV)infection.Methods Sixty-two patients with CCA who underwent patho-logical examination in this hospital from January 2021 to September 2022 were selected as the CCA group,65 patients with cervical intraepithelial neoplasia(CIN)as the CIN group,and 63 patients with normal cervix as the control group.The positive expression rates of cervical STING and ZEB1 and high-risk HPV infection in each group were observed and recoraded.The correlation of STING and ZEB1 levels with clinicopathological features of CCA and high-risk HPV infection was analyzed.Results The positive expression rates of STING and ZEB1 in CCA group were higher than those in CIN group and control group,and the level of ZEB1 in CIN group was higher than that in control group,with statistical significance(P＜0.05).The detection rates of HPV16 and 18 in CCA group were higher than those in CIN group and control group,and the detection rates of HPV16 and 18 in CIN group were higher than those in control group,the differences were statistically sig-nificant(P＜0.05).The infiltration depth and FIGO stage of CCA patients were correlated with the positive expression rates of STING and ZEB1,the degree of tumor differentiation and lymph node metastasis were cor-related with the positive expression rates of STING(P＜0.05),and the positive expression rates of STING and ZEB1 in CCA patients were positively correlated with the infection rates of HPV16 and 18(P＜0.05).Conclusion The positive expression levels of STING and ZEB1 proteins in the CCA group are higher than those in the control group and CIN group,and their expression levels were related to FIGO staging and depth of cervical cancer infiltration.STING and ZEB1 proteins are positively correlated with HPV16 and 18 infec-tion,and STING and ZEB1 proteins may jointly act with HPV16 and 18 on the occurrence and development of CCA.

Hypertension (HT) can induce aortic dissection (AD) by increasing the tension of the aortic wall and promoting tissue degradation, and continuous HT may increase the risk of aortic-related death. It is a significant factor in poor prognosis for AD and the most important controllable factor. Research has shown that blood pressure management after AD can reduce the incidence rate and mortality of AD, so controlling HT is an important goal and means of AD treatment. This article reviews the research progress on the impact of AD on blood pressure, the significance and methods of blood pressure management after AD, with the hope of providing reference for the treatment of AD patients and benefiting them.

Objective:To evaluate the effect of controlled low central venous pressure (CLCVP) induced by milrinone on intraoperative cerebral blood flow in the patients undergoing laparoscopic hepatectomy.Methods:Eighty American Society of Anesthesiologists Physical Status classification Ⅰ-Ⅲ patients, regardless of gender, scheduled for elective laparoscopic hepatectomy, with body mass index of 18-30 kg/m 2, aged 18-64 yr, were divided into 2 groups ( n=40 each) by a random number table method: nitroglycerin group (X group) and milrinone group (M group). After the start of surgery, milrinone 0.5 μg·kg -1·min -1 was continuously infused in group M and nitroglycerin 0.5-1.5 μg·kg -1·min -1 was continuously infused in group X to maintain central venous pressure (CVP)<5 cmH 2O. Norepinephrine 0.01-0.10 μg·kg -1·min -1 was intravenously injected during operation to maintain mean arterial pressure at 60-90 mmHg. Norepinephrine 8-16 μg was intravenously injected when necessary. At 10 min after tracheal intubation (T 1), after establishment of pneumoperitoneum (T 2), at 5 and 30 min after CVP reached the target value (T 3, T 4), at 5 min after CVP returned to normal value (T 5) and at the end of surgery (T 6), the internal carotid artery mean blood flow velocity, internal carotid artery blood flow, cardiac output, and cardiac index were recorded. The surgical field score, consumption of intraoperative norepinephrine, fluid input and output, and occurrence of adverse reactions at 7 days after operation were recorded. Results:Compared with group X, the cardiac output, cardiac index, internal carotid artery mean blood flow velocity and internal carotid artery blood flow were significantly increased, the consumption of intraoperative norepinephrine was decreased, and the surgical field score was increased at T 3-5 in group M ( P<0.05). There was no significant difference in the intraoperative fluid intake and output and incidence of postoperative adverse reactions at each time point between the 2 groups ( P>0.05). Conclusions:Compared with nitroglycerin-induced CLCVP, milrinone-induced CLCVP can improve the reduction in intraoperative cerebral blood flow in the patients undergoing laparoscopic hepatectomy to some extent.

Objective:To explore the method of constructing automatic delineation model for clinical target volume (CTV) and partially organs at risk (OAR) of postoperative radiotherapy for prostate cancer based on convolutional neural network, aiming to improve the clinical work efficiency and the unity of target area delineation.Methods:Postoperative CT data of 117 prostate cancer patients manually delineated by one experienced clinician were retrospectively analyzed. A multi-class auto-delineation model was designed based on 3D UNet. Dice similarity coefficient (DSC), 95% Hausdorf distance (95%HD), and average surface distance (ASD) were used to evaluate the segmentation ability of the model. In addition, the segmentation results in the test set were evaluated by two senior physicians. And the CT data of 78 patients treated by other physicians were also collected for external validation of the model. The automatic segmentation of these 78 patients by CTV-UNet model was also evaluated by two physicians.Results:The mean DSC for tumor bed area (CTV1), pelvic lymph node drainage area (CTV2), bladder and rectum of CVT-UNet auto-segmentation model in the test set were 0.74, 0.82, 0.94 and 0.79, respectively. Both physicians' scoring results of the test set and the external validation showed more consensus on the delineation of CTV2 and OAR. However, the consensus of CTV1 delineation was less.Conclusions:The automatic delineation model based on convolutional neural network is feasible for CTV and related OAR of postoperative radiotherapy for prostate cancer. The automatic segmentation ability of tumor bed area still needs to be improved.

OBJECTIVE: To explore the clinical characteristics and genetic basis of two brothers featuring X-linked alpha thalassemia mental retardation (ATR-X) syndrome. METHODS: An infant who had presented at the Qilu Children's Hospital in 2020 for unstable upright head and inability to roll over and his family were selected as the study subjects. The clinical features of the child and one of his brothers were summarized, and their genomic DNA was subjected to targeted capture and next generation sequencing (NGS). RESULTS: The brothers had presented with mental retardation and facial dysmorphisms. NGS revealed that they had both harbored a hemizygous c.5275C>A variant of the ATRX gene located on the X chromosome, which was inherited from their mother. CONCLUSION The siblings were diagnosed with ATR-X syndrome. The discovery of the c.5275C>A variant has enriched the mutational spectrum of the ATRX gene.
Humans ; Infant ; Male ; alpha-Thalassemia/diagnosis* ; Ataxia Telangiectasia Mutated Proteins/genetics* ; East Asian People ; Intellectual Disability/genetics* ; Mental Retardation, X-Linked/diagnosis* ; Pedigree ; X-linked Nuclear Protein/genetics*

Chronic alcohol consumption causes liver steatosis, cell death, and inflammation. Melatonin (MLT) is reported to alleviate alcoholic liver disease (ALD)-induced injury. However, its direct regulating targets in hepatocytes are not fully understood. In the current study, a cell-based screening model and a chronic ethanol-fed mice ALD model were used to test the protective mechanisms of MLT. MLT ameliorated ethanol-induced hepatocyte injury in both cell and animal models (optimal doses of 10 μmol/L and 5 mg/kg, respectively), including lowered liver steatosis, cell death, and inflammation. RNA-seq analysis and loss-of-function studies in AML-12 cells revealed that telomerase reverse transcriptase (TERT) was a key downstream effector of MLT. Biophysical assay found that epidermal growth factor receptor (EGFR) on the hepatocyte surface was a direct binding and regulating target of MLT. Liver specific knock-down of Tert or Egfr in the ALD mice model impaired MLT-mediated liver protection, partly through the regulation of nuclear brahma-related gene-1 (BRG1). Long-term administration (90 days) of MLT in healthy mice did not cause evident adverse effect. In conclusion, MLT is an efficacious and safe agent for ALD alleviation. Its direct regulating target in hepatocytes is EGFR and downstream BRG1-TERT axis. MLT might be used as a complimentary agent for alcoholics.

Objective:To evaluate the effects of controlled low central venous pressure (CLCVP) on the cerebral blood flow in the patients undergoing open hepatectomy.Methods:Thirty American Society of Anesthesiologists Physical Status classification Ⅱ or Ⅲ patients of either sex, aged 18-60 yr, with body mass index of 18-30 kg/m 2, with Child-Pugh classification A and expected operation time of 2-4 h, undergoing elective open hepatectomy, were enrolled.After anesthesia induction, patients were placed at head-up tilt position, nitroglycerin 0.5-1.5 μg·kg -1·min -1 was infused, and furosamide 5-10 mg was intravenously injected when necessary to maintain CVP less than 5 cmH 2O during hepatectomy.After the end of hepatectomy, CLCVP was stopped, the infusion rate was increased to 10 ml·kg -1·h -1 with a crystalline gel ratio of 1∶2 to restore CVP to more than 5 cmH 2O.At 5 min after anesthesia induction (T 0), 5 min after head-up tilt (T 1), 5 and 15 min after CVP reaching the target (T 2, 3) and 5 min after the end of CLCVP (T 4), the blood flow of internal carotid artery was detected by ultrasound, and peak systolic velocity(IBVs), end diastolic velocity (IBVd)and vessel diameter of the internal carotid artery (ID)were measured by doppler ultrasound.Mean velocity [IBVm=(IBVs+ IBVd×2)÷3] and internal carotid artery blood flow [IBF=IBVm×π×(ID/2) 2×HR] were calculated.Heart rate (HR), mean arterial blood pressure (MAP), cardiac output (CO), stroke volume (SV), end-tidal pressure of carbon dioxide (P ETCO 2) and maximum airway pressure (P max) were recorded at each time point. Results:Compared with the baseline at T 0, MAP, CO, SV, IBVm and IBF were significantly decreased at T 2, 3 ( P<0.001), and no significant change was found in HR, P ETCO 2 and P max at T 1-4 ( P>0.05). The results of linear mixed-effects model analysis showed that the regression coefficients for CO, MAP, HR, and SV were 0.600 3, 0.022 88, 0.363 7, and 0.614 8, respectively ( P<0.05 or 0.01). Conclusions:CLCVP can decrease the cerebral blood flow in the patients, which is closely associated with decreased CO, MAP, HR and SV when used for open hepatectomy.